Cardiac amyloidosis is an infiltrative, restrictive, cardiomyopathy characterized by right and left ventricular thickening, due to extracellular deposition of amyloid. The myocardial infiltration in amyloidosis results in distortion and replacement of myocytes, with an associated decrease in the voltage of the 12-lead electrocardiogram, particularly in the limb leads. The term "restrictive cardiomyopathy" refers to the finding of non-dilated left ventricle associated with severely impaired left ventricular filling caused by an extremely stiff ventricle. This is visualized on Doppler echocardiography as a rapid deceleration of transmitral flow during early diastolic filling and a reduced flow during late diastole. …

AbstractObjectives: To determine how often left ventricular wall thickness (LVWT) is normal and to assess the effect of LVWT on clinical outcomes of patients with immunoglobulin light chain (AL) cardiac amyloidosis.Patients and Methods: A total of 117 patients with systemic AL amyloidosis were…

Alnylam Pharmaceuticals has initiated the Endeavour Phase 3 clinical trial of revusiran in transthyretin (TTR)-mediated familial amyloidotic cardiomyopathy (FAC). According to a release, FAC is one of the predominant clinical manifestations of TTR-mediated amyloidosis (ATTR), and afflicts an…

Jon T. Hoffman knew something was very wrong with him, even if his doctors didn't. Shortly after returning to Northern Virginia from a three-week stint in Iraq in August 2003, the 47-year-old Marine Corps reservist and military historian developed an itchy rash on his ankle, then his face. That was…